WHITE DOT SYNDROMES

1. WHITE DOT SYNDROMES
Dr RAKHIDCRUZ

2. WHITE DOT SYNDROMES
• Birdshot choroidopathy
• Multifocal Choroiditis and Panuveitis (MCP)
• Multiple Evanescent White Dot Syndrome (MEWDS)
• Subretinal fibrosis and uveitis(SFU)
• Acute Zonal Occult Outer Retinopathy (AZOOR)
• Punctate Inner Choroidopathy( PIC)
• Acute Idiopathic Blind Spot Enlargement Syndrome (AIBES)
• Diffuse unilateral subacute neuroretinitis(DUSN)
• Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
• Seripigenous Choroidopathy

3. WHITE DOT SYNDROMES in general…
Group of diseases characterized by inflammation and dysfunction of the outer retina, retinal
pigment epithelium, choroid, or a combination of these.
WHITE DOT SYNDROMES etiology
Unknown – Infectious – Autoimmune/ Inflammatory
May be inherited Immune dysregulation that predisposes to Autoimmunity
Symptoms-Blurred vision, photopsias, visual field changes, floaters and changes in contrast
sensitivity
Bilateral Involvement although asymmetrical (except MEWDS) is a RULE

4. • • Majority of patients are younger than 50 y/0 (except Birdshot
Retinochoroidopathy & Serpiginous Choroiditis
• • Female Preponderance in Birdshot choroidopathy, PIC, MCP,
AZOOR &MEWDS

5. BIRDSHOT RetinoChoroidopathy
• HLA-A29 is 96% Sensitive
• Multifocal, Hypopigmented Ovoid Cream lesions (50-1500 um)
• Symptoms- Blurring of vision, floaters, Nyctalopia
• Inflammation is primary feature
• Vitritis-Common but variable severity
• Retinal vasculitis and CME
• CNV- Rare
• Vitiliginous ChorioRetinitintis

6. • FFA Findings -Early hypo/late diffuse hyper,Doesn't’t typically highlight
spots
• ICGA Findings Shows hypoautofluorescent spots bordered by large
vessels- More numerous on exam
• ERG
• Delayed 30 Hz Flicker Implicit Time Diminished scotopic B wave
amplitudes
• Treatment: Initially- coticosteroids
• immunosuppresives
• ANTI-VEGF agents(CNV)

7. Acute Posterior Multifocal Placoid Pigment
Epitheliopathy (APMPPE)
• Young middle aged adults of both genders
• An antecedent viral prodrome occurs(50%), and it is speculated to occur
because of cell
mediated immunity to viral antigen.(mumps, adenovirus, coxsackivirus)
• Symptoms: BOV, Scotoma, Photopsias
• Vitritis: Mild to moderate in 50%
• CNV: Rare
• HLAB27&HLADR2

8. Fundoscopy: Multiple large
flat,yellow-white placoid
lesions at the level of RPE.
They are 1-2 disc diameters
in size and are located
throughout the posterior
pole.
• Acute lesions heal over a
period of 2-6 weeks with
RPE pigmentary alterations
&
resultant chorioretinal
atrophy

9. A P M P P E Associations
Non-Infetious
• Erythema Nodosum
• Wegener’s Granulomatosis
• Polyarthritis Nodosa
• Cerebral Vasculitis
• Scleritis & Episcleritis
• Ulcerative Colitis
Infectious
• Group A Streptococcus
• Adenovirus Type 5
• TB
• Lyme Disease
• Mumps
• Hepatitis B vaccination

10. • FA Findings Blocks early, Stains Late
• ICGA Findings Hypofluorescent Spots= No. in FA
• AF Findings HypoAF lesions
• The early hypofluorescence- IVFA and ICG suggest that
nonperfusionor infarction of the choroid, perhaps secondary to
vasculitis,may be the primary disorder. Associations withsystemic
vasculitides support this hypothesis
• APMPPE PROGNOSIS-GOOD with 20/40 or better in 3-6 weeks
RISK FACTORS FOR VISION LOSS
FOVEAL Involvement
Older Age
Unilateral Disease
Longer interval of the initial and fellow eye involvement
Recurrence

11. Serpiginous Choroiditis
• Asymmetric Gray White lesions at the level of the RPE in a Pseudopodial
/Geographic manner from the Optic Nerve
• Both sex 2 -7th decade
• bilateral, chronic, and progressive inflammatory
Symptoms: BOV, Scotomas
Signs-Vitritis mild
Geographic Helicoid perpapillary Choroidopathy
Late complications include retinal vein occlusion, macular hole, subretinal fibrosis
and CNV usually occurring at the border of an old scar

12. • Fundoscopy
• Serpiginous Choroiditis
• FA Findings: Blocks early, Stains Late (*plus the CNV findings if
Present)
• ICG Findings: Hypofluorescent lesions throughout
• FAF : Active lesions are HyperAF; inactive Lesions are HypoAF
• RX- SYSTEMIC STEROIDS + IMMUNOSUPRESSANTS

15. Multifocal Choroiditis and Panuveitis
• Myopic women between second and third decade of life
• Photopsia, decreased vision, floaters, photophobia
• Bilateral but may be asymmetric
• The etiology is not known but may be due to sensitization of antigens within
photoreceptors & retinal pigment epithelial cells by an exogenous pathogen
(epstein barr virus or hsv)
• signs- anterior uveitis, vitritis, multifocal choroiditis.

16. • The choroiditis lesions are 50-200 microm in diameter, yellow-
white at the level of RPE or inner choroid and have punched out
appearance with pigmentation of the edges
FA findings : acute-Early Hypoflurescence ,Late
staining for active lesions
ICGA findings: Hypofluorescent Spots are more
numerous than FA
FAF: Active lesions are HyperAF; Inactive lesions
HypoAF

17. • Rx-
• • topical, systemic steroids. In chronic cases immunosuppressive
agents can beconsidered
• • CNV can be managed with anti-VEGF therapy
,steroids,photodynamic therapy

18. Punctate Inner Choroidopathy (PIC)
• Young Myopic Female 2/3
• Symptoms: Photopsia, Metamophopsia, BV
• Mild anterior uveitis/vitritis.
• lesions are bilateral, multiple, small,welldefined, yellow-white, usually 100-200
microns diameter and are limited to the posterior pole mostly macular
• CNV YES!; 79% At Presentation,recurrence common
• FA Findings: Early Hyperfluorescence, late staining
• ICGA Findings: Hypofluorescent spont = FA
• TREATMENT Steroids topical & systemic
• CNVM-Anti VEGF/Laser Photocoagulation/PDT

19. MULTIPLE EVANESCENT WHITE DOT SYNDROME
(MEWDS)
• Young Myopic Female
• Symptoms: ACUTE UNILATERAL Photopsias, dyschromatopsia, BV
• Anterior segment within normal limits
• VITRITIS: Variable,optic disc edema can occur
• Circumpapillary patches,paramacular involvement &CNV Rarely
• FUNDOSCOPY: ACUTE PHASE Multiple discrete white to orange Spots
(100-200um) at the level of the RPE or Deep retina.
• “EVANESCENT” Because those spots are TRANSITORY
• and frequently MISSED

20. • ORANGE GRANULAR PIGMETARY CHANGE – macula
• HFA-enlargement of blindspot,central and paracentral scotomas
• FA FINDINGS :
• Punctate HYPER fluorescent spots that surrounds the fovea in a wreath-like
configuration
• ICGA FINDINGS:multiple small, round hypofluorescen spots in the posterior and
midperipheral fundus. The number of spots seen on ICG may be more numerous
• ERG FINDINGS: Diminished A-Wave
• MEWDS resolves spontaneously without need for treatment.
• The prognosis is generally very good

21. Sub-Retinal Fibrosis and Uveitis Syndrome
• is a rare form of panuveitis of unknown etiology
• healthy myopic women between 14 and 34 years
• Symptoms – blurring of vision in one eye then later both eyes
• Signs- mild to moderate vitritis, with whiteyellow lesions (50-500 μm)
located in the posterior pole to midperiphery at the level of the RPE
• These lesions are accompanied by the appearance of turbid subretinal
exudation & this differentiates SFU from others like MFC & PIC,these
exudtaes later coalesce into large, white, stellate zones of subretinal
fibrosis to involve most of the retina and choroid

22. Treatment is directed towards early
diagnosis and aggressive management
to prevent fibrosis setting in the other
eye.
Once severe subretinal fibrosis
develops, treatment has little benefit

23. WHITE DOT SYNDROME DIFFERENTIAL
• Syphilis
• Diffuse Unilateral Subacute Neuroretinitis
• Ocular Histoplasmosis Syndrome
• Tuberculosis
• Sarcoidosis
• Sympathetic Ophthalmia
• VKH
• Intraocular Lymphoma
• Pneumocystis Choroidopathy
• Candidiasis